is an impaired immune system
most common symptomatic primary immunodeficiency
caused by
mostly unknown
<10% genetic
resulting in
recurrent respiratory tract infections.
Cellular changes
serum IgA and IgG: decreased but not absent
serum IgM: occasionally decreased
circulating T and B lymphocytes: present
Organs
skin
sinuses
lung
bronchiectasis withILD
(e.g. granulomatous-lymphocytic interstitial lung disease )
recent term given to describe non-infectious diffuse lung disease complications that have been reported to traditionally develop in common variable immunodeficiency (CVID) patients
histologic patterns
bronchiectasis could either be due to repeated lung infection or manifestation of dysregulated lymphoid proliferation 1
bronchiectasis has been reported as up to 65% of patients according to one study 2
~10% of patients a systemic granulomatous disease
pulmonary nodules
ground glass changes
lung parenchymal opacification: may be multifocal
other features
intrathoracic or intra-abdominal lymphadenopathy and/ or hepatosplenomegaly
liver
hematologic
TCV
TCV
